Mixed Neuroendocrine and Non-Neuroendocrine Neoplasm of Pancreas: What Do We Know, What Have We Learnt?

Pancreatic adeno-mixed neuroendocrine non-endocrine (pMINEN) tumors are extremely rare [Pancreatology. 2021;21(1):224-235]. They are known to have distal metastasis at presentation and have a comparatively lower survival rate than similar staged neuroendocrine (NEN) carcinoma, adenocarcinoma, and small-cell lung tumor from which its treatment patterns are extrapolated. Also, very less is known about its molecular structure and natural courses. There is a dearth of data about pMINEN in the literature, and also there is a lack of large multicentral trials due to which the MINEN tumors do not have a standard universal management protocol. We discuss here the clinical dilemmas that arise during diagnosis and reporting and urge to form a multicentric trial to formulate a focused protocolized approach. We describe here our encounter with a pancreatic head lesion which on immunohistochemical analysis turned out to be a pMINEN with moderately differentiating ductal adenocarcinoma and low-grade NEN tumor. Radical R0 surgery with multimodal treatment (chemotherapy + radiotherapy) gains improved survival in long term.

Gallbladder Neuroendocrine Tumors: Is There a Need for a Specific Approach?

Neuroendocrine tumors (NETs) of the gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing lung small cell neuroendocrine carcinoma or gastrointestinal NETs. But, the overall outcome of gallbladder NETs (GB-NETs) seems worse than similarly staged lung NETs and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach toward its treatment. Hence, the need for a specifically designed approach might help improve results of treatment for these rare tumors. We share our experience of 2 patients with GB-NETs and their 5-year outcome.

Successful Use of Direct Splenic Vein Anastomosis to the Interposition Internal Jugular Vein Graft after Extended Pancreatoduodenectomy to Avoid Sinistral Portal Hypertension.

Splenic vein (SV) ligation may be needed during portomesenteric junction resection, in pancreatoduodenectomy. Sinistral portal hypertension is a concern if the SV is not drained. Various techniques are described to reconstruct SV to avoid the variceal formation and sinistral portal hypertension which may lead to GI bleed. We describe a case of a 19-year-old female who underwent pancreatoduodenectomy for solid pseudopapillary neoplasm with portal-superior mesenteric vein junction resection and splenic venous was anastomosed into the interposition graft. We here share our unique experience of using an interposition internal jugular vein graft for a long venous defect and diverging morbidity of sinistral portal hypertension.

Idiopathic CD4 Lymphocytopenia Presenting as Cryptococcal Meningitis.

A 54-year-old male presented to our center with a 3-month history of headache, giddiness, and blurring of vision. Cerebrospinal fluid examination revealed him to be having cryptococcal meningitis. He was worked up for probable causes of immunosuppression including HIV and other infections and had an autoimmune profile as well as a bone marrow examination, none of which revealed any abnormality. Lymphocyte flow cytometry revealed low counts of CD4 T lymphocytes, likely secondary to idiopathic CD4 lymphocytopenia. He was treated for cryptococcal meningitis. Due to marked immunosuppression, the disease progressed rapidly with deterioration in neurological and hemodynamic status, leading to his demise.

Ileal cavernous haemangioma in an adult presenting as a rare cause of small bowel obstruction.

Haemangiomas of the small bowel are a very rare entity and rarely considered as an aetiology for an intestinal obstruction. Contrast-enhanced CT is the investigation of choice but the lesion can be confused with malignancy or rarely a neuroendocrine tumour. Commonly it presents as abdominal pain with anaemia and/or melaena. With patients presenting without obstruction or acute gastrointestinal bleed, capsule endoscopy has shown to be a useful diagnostic tool.We present here our experience of managing a case of a patient with ileal haemangioma who presented with a subacute small bowel obstruction and underwent a laparoscopic-assisted ileal segmental resection with side-to-side anastomosis. The lesion was a cavernous haemangioma on histopathological analysis.

New Onset Refractory Status Epilepticus as a Manifestation of Tuberculosis of the Central Nervous System.

A 28-year-old male presented to us with new onset refractory status epilepticus. Prior to his seizures, he had a history of fever, headache and blurring of vision, for which he was admitted elsewhere. No cause was found; he was treated symptomatically and was discharged as his symptoms had abated. Post-discharge, he started having multiple seizures. Neuro-imaging suggested encephalitis. He progressed to status epilepticus and was referred to our center. We intensified the anti-epileptic medications but owing to no response, he had to be put under coma with thiopental, yet the seizures persisted. Workup for the etiology of his seizures was negative except for CSF TB-PCR (Gene Xpert) being positive and hence anti-tuberculous therapy was initiated. By this time, he developed rhabdomyolysis, and thereafter renal failure with dyselectrolytemia, and thus there was a plan to initiate dialysis. But before this could be done, he succumbed to a cardiac arrest secondary to ventricular tachyarrhythmias. We believe this to be the first reported case of new onset refractory status epilepticus secondary to tuberculosis of the central nervous system.

Handlebar sign: a bruise with underlying visceral injury.

A 14-year-old boy presented with a painful swelling topped by a bruise overlying the skin of the right inguinal region without peritonitis. This was the area of impact of bicycle handlebar while riding 6 days ago. On contrast-enhanced CT scan, we found a traumatic abdominal wall hernia (handlebar hernia) near the right deep ring without any solid organ, bowel or urinary bladder injury. Inguinal exploration revealed a defect in transversus abdominis and internal oblique muscle which was repaired and meshplasty was done.Delayed presentation and ignorance towards 'handlebar sign' is associated with visceral injury (haematoma/perforation) will incur the risk of rising morbidity and mortality. With CT scan we can assess the abdominal cavity to rule out associated visceral or vascular injury. Surgical repair for restoring disrupted anatomy with or without meshplasty is the preferred approach.

Dysautonomia and hyponatraemia as harbingers of Guillain-Barre syndrome.

A 56-year-old woman with a medical history of hypertension presented to our hospital with back pain, abdominal pain, vomiting and elevated blood pressure. The laboratory parameters including evaluation for secondary hypertension were within normal ranges at the time of presentation. During her hospitalisation, fluctuations in her blood pressure and pulse were observed which were attributed to autonomic disturbances, the cause of which was unknown. On the seventh day after presentation to the hospital, the patient developed focal seizures and slurred speech which was believed to be secondary to hyponatraemia detected at that time. Hyponatraemia improved with hypertonic saline and she experienced no further seizures. On the eighth day of her admission, she developed acute flaccid paralysis of all her limbs and respiratory distress. We concluded this to be secondary to Guillain-Barre syndrome (GBS). She responded to plasmapheresis.The presence of dysautonomia and hyponatraemia before the onset of paralysis makes this a rare presentation of GBS.

'Little old lady's hernia' (obturator hernia): A deceptive encounter.

Obturator hernia (OH) is rare which not only carries high mortality amongst all abdominal hernia, but also known for the difficulty in diagnosing it. Howship-Romberg sign is a clinical sign to diagnose OH, but due to the lower-limb muscle contractures, it was not possible in our case. Computed tomography scan becomes the investigation of choice in this situation. A laparoscopic approach can be used safely.

Successful treatment of extensive splanchnic vein thrombosis in a patient with mycosis fungoides.

A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.